Congenital pulmonary sequestration

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Synonyms: Congenital bronchopulmonary sequestration

Congenital pulmonary sequestration is a rare respiratory malformation characterized by a cystic or solid mass of nonfunctioning primitive segmental lung tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. Intralobar pulmonary sequestration may be asymptomatic or may present with recurrent pulmonary infections hemoptysis chest pain cough and is usually diagnosed in older children and adults. Extralobar pulmonary sequestration present with respiratory distress cyanosis difficulty feeding or infection may be associated with other anomalies and is mostly diagnosed in neonates or infants.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version May 2024

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